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Gastrointestinal Involvement in IgG4-Related Disease

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Gastrointestinal and Hepatic Manifestations of Rheumatic Diseases

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Abstract

IgG4-related disease (IgG4-RD) is characterized by increased serum IgG4 and swelling of several organs or nodules by infiltration of IgG4-positive plasma cells and lymphocytes, as well as fibrosis observed throughout the body. In the gastrointestinal area, autoimmune pancreatitis (AIP) and IgG4-related sclerosing cholangitis (IgG4-SC) are primarily observed. In 2010, the International Consensus Diagnostic Criteria (ICDC) for AIP were proposed, and in 2012, clinical diagnostic criteria for IgG4-SC were proposed. Nevertheless, the aetiology of IgG4-RD remains unknown, and histological diagnosis of gastrointestinal IgG4-RD remains difficult. Differentiation between gastrointestinal IgG4-RD and malignant pancreaticobiliary diseases is difficult. Steroids have become the established therapy for IgG4-RD; however, predictive relapse factors are controversial. In this chapter, we introduce the history, diagnosis and treatment of gastrointestinal IgG4-RD, as well as several challenges to ameliorating the difficulties mentioned above.

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Authors and Affiliations

  1. Department of Gastroenterology, Fukushima Medical University School of Medicine, Fukushima, Japan

    Mitsuru Sugimoto & Hiromasa Ohira

  2. Department of Diagnostic Pathology, Fukushima Medical University School of Medicine, Fukushima, Japan

    Erina Suzuki, Kazuhiro Tasaki & Yuko Hashimoto

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  1. Mitsuru Sugimoto
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  2. Erina Suzuki
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  3. Kazuhiro Tasaki
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  4. Yuko Hashimoto
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  5. Hiromasa Ohira
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Correspondence to Mitsuru Sugimoto .

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  1. Department of Gastroenterology, Fukushima Medical University School of Medicine, Fukushima, Japan

    Hiromasa Ohira

  2. Department of Rheumatology, Fukushima Medical University School of Medicine, Fukushima, Japan

    Kiyoshi Migita

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Sugimoto, M., Suzuki, E., Tasaki, K., Hashimoto, Y., Ohira, H. (2019). Gastrointestinal Involvement in IgG4-Related Disease. In: Ohira, H., Migita, K. (eds) Gastrointestinal and Hepatic Manifestations of Rheumatic Diseases. Springer, Singapore. https://doi.org/10.1007/978-981-13-6524-9_11

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